Clinical Characteristics of 76 Patients with IgG4-Related Hypophysitis: A Systematic Literature Review

Background. IgG4-related hypophysitis (IgG4-RH) is a rare disease, and its prevalence remains unclear. In recent years, an increasing number of cases have been reported because of the increasing recognition of this disease. We aimed to summarize case reports of IgG4-RH and outline the clinical features and outcomes. Methods. We performed PubMed search of articles using the search terms “hypophysitis [AND] IgG4.” Consequently, only 54 English articles (76 cases) met Leporati’s diagnostic criteria. Results. Of the 76 cases, the ratio of men to women was 1.5 : 1, and the age at diagnosis was 54.1 ± 17.8 years. The median IgG4 concentration was 405.0 mg/dl. Anterior hypopituitarism, isolated central diabetes insipidus, and panhypopituitarism were observed in 14 (18.4%), 12 (15.8%), and 44 (57.9%) cases, respectively. The sequence of anterior hormone deficiency was as follows: gonadotropin (68.4%), ACTH (63.2%), TSH (59.2%), GH (48.7%), and prolactin (42.1%). The median number of involved organs was 1.5, and the lung (18.4%), retroperitoneum (17.1%), kidney (15.8%), submandibular glands (14.5%), and pancreas (13.2%) were the common involved organs. Elevated IgG4 concentration and normal IgG4 level were in 42 (76.4%) and 13 (23.6%) cases, respectively. Patients with elevated serum IgG4 concentration were older (60.9 ± 14.3 vs 45.6 ± 17.4, p=0.001) and male-prone (78.6% vs 40.4%, p=0.003) and had a susceptibility of multiple organ involvement (78.6% vs 35.0%, p=0.001) compared to those with normal serum IgG4 levels. Males were older at disease onset (61.5 ± 12.6 vs 42.9 ± 18.8, p<0.001) and had a higher IgG4 concentration (425.0 vs 152.5, p=0.029) and a greater number of involved organs (2.0 vs 0.0, p=0.001), while isolated hypophysitis was more prominent in female (63.3% vs 26.1%, p=0.001). Conclusion. In this review, we found that there were different characteristics between different genders. Patients with elevated serum IgG4 level in terms of some clinical features were also different from those with normal serum IgG4 level. However, the data in this review were limited by bias and confounding. Further clinical studies with larger sample sizes are warranted.

Download Full-text

IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia

Case Reports in Nephrology and Dialysis ◽

10.1159/000500296 ◽

2019 ◽

Vol 9 (2) ◽

pp. 49-54

Author(s):

Emmett Tsz Yeung Wong ◽

Manjari Lahiri ◽

Ming Teh ◽

Christopher Cheang Han Leo

Keyword(s):

Kidney Disease ◽

Interstitial Nephritis ◽

Elevated Serum ◽

Kidney Injury ◽

Acute Interstitial Nephritis ◽

Clinical Syndrome ◽

Normal Serum ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

IgG4-related kidney disease has been relatively newly recognized over the last two decades as a combination of an autoimmune and allergic disorder, with elevated serum IgG4 level and hypocomplementemia among its characteristic features. Here we report the case of a man with interstitial nephritis presenting with acute kidney injury and hypocomplementemia but normal serum IgG4 level and provide a literature review of IgG4-related kidney disease. This case highlights the importance of IgG4-related kidney disease as an important differential diagnosis in any patient presenting with a clinical syndrome mimicking acute interstitial nephritis with hypocomplementemia. A high index of suspicion with a low threshold for performing a native kidney biopsy would be paramount as patients do respond well to corticosteroid therapy.

Download Full-text

Hepatobiliary and Pancreatic: Pancreatic cancer with elevated serum IgG4 level due to multiple myeloma mimicking localized autoimmune pancreatitis

Journal of Gastroenterology and Hepatology ◽

10.1111/jgh.14088 ◽

2018 ◽

Vol 33 (7) ◽

pp. 1310-1310 ◽

Cited By ~ 3

Author(s):

S Kato ◽

M Kuwatani ◽

K Kawakubo ◽

R Sugiura ◽

K Hirata ◽

...

Keyword(s):

Pancreatic Cancer ◽

Multiple Myeloma ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

Download Full-text

Spectrum of Disorders Associated with Elevated Serum IgG4 Levels Encountered in Clinical Practice

International Journal of Rheumatology ◽

10.1155/2012/232960 ◽

2012 ◽

Vol 2012 ◽

pp. 1-6 ◽

Cited By ~ 54

Author(s):

Jay H. Ryu ◽

Ryohei Horie ◽

Hiroshi Sekiguchi ◽

Tobias Peikert ◽

Eunhee S. Yi

Keyword(s):

Clinical Practice ◽

Wide Spectrum ◽

Systemic Vasculitis ◽

Elevated Serum ◽

Serum Igg4 Level ◽

Igg4 Related Disease ◽

Clinical Presentations ◽

Serum Igg4 ◽

Disease Associations ◽

Igg4 Level

IgG4-related disease (IgG4-RD) is a recently described systemic fibroinflammatory disease associated with elevated circulating levels of IgG4 and manifests a wide spectrum of clinical presentations. Although serum IgG4 level has been described to be the most sensitive and specific laboratory test for the diagnosis of IgG4-RD, it is recognized that an elevated serum IgG4 level can be encountered in other diseases. In this study, we sought to identify the frequency of IgG4-RD and other disease associations in patients with elevated serum IgG4 levels seen in clinical practice. Among 3,300 patients who underwent IgG subclass testing over a 2-year period from January 2009 to December 2010, 158 (4.8%) had an elevated serum IgG4 level (>140 mg/dL). IgG4 subclass testing was performed for evaluation of suspected IgG4-RD or immunodeficiency. Twenty-nine patients (18.4%) had definite or possible IgG4-RD. Among those patients without IgG4-RD, a broad spectrum of biliary tract, pancreatic, liver, and lung diseases, as well as systemic vasculitis, was diagnosed. We conclude that patients with elevated serum IgG4 levels encountered in clinical practice manifest a wide array of disorders, and only a small minority of them has IgG4-RD.

Download Full-text

Serum Immunoglobulin G Fraction 4 Levels in Pancreatic Cancer: Elevations Not Associated With Autoimmune Pancreatitis

Archives of Pathology & Laboratory Medicine ◽

10.5858/2008-132-48-sigfli ◽

2008 ◽

Vol 132 (1) ◽

pp. 48-53 ◽

Cited By ~ 8

Author(s):

Amit Raina ◽

Alyssa M. Krasinskas ◽

Julia B. Greer ◽

Janette Lamb ◽

Erin Fink ◽

...

Keyword(s):

Pancreatic Cancer ◽

Cancer Patients ◽

Immunoglobulin G ◽

Autoimmune Pancreatitis ◽

Elevated Serum ◽

Serum Immunoglobulin ◽

Pancreatic Mass ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

Abstract Context.—Autoimmune pancreatitis is an uncommon, inflammatory disease of the pancreas that presents with clinical features, such as painless jaundice and a pancreatic mass, similar to those caused by pancreatic cancer. Patients with autoimmune pancreatitis frequently have elevated serum immunoglobulin G fraction 4 (IgG4) levels, and their pancreatic tissue may show IgG4-positive plasma cell infiltration. It is imperative to differentiate autoimmune pancreatitis from pancreatic cancer because autoimmune pancreatitis typically responds to corticosteroid treatment. A previous Japanese study reported that serum IgG4 greater than 135 mg/dL was 97% specific and 95% sensitive in predicting autoimmune pancreatitis. Objective.—To prospectively measure serum IgG4 levels in pancreatic cancer patients to ascertain whether increased levels might be present in this North American population. Design.—We collected blood samples and phenotypic information on 71 consecutive pancreatic cancer patients and 103 healthy controls who visited our clinics between October 2004 and April 2006. IgG4 levels were determined using a single radial immunodiffusion assay. A serum IgG4 level greater than 135 mg/dL was considered elevated. Results.—Five cancer patients had IgG4 elevation, with a mean serum IgG4 level of 160.8 mg/dL. None of our cancer patients with plasma IgG4 elevation demonstrated evidence of autoimmune pancreatitis. One control subject demonstrated elevated serum IgG4 unrelated to identified etiology. Conclusions.—As many as 7% of patients with pancreatic cancer have serum IgG4 levels above 135 mg/dL. In patients with pancreatic mass lesions and suspicion of cancer, an IgG4 level measuring between 135 and 200 mg/dL should be interpreted cautiously and not accepted as diagnostic of autoimmune pancreatitis without further evaluation.

Download Full-text

Elevated Serum IgG4 Level is a Poor Predictor of the Diagnosis of IgG4-Related Disease

The American Journal of Gastroenterology ◽

10.14309/00000434-201210001-00245 ◽

2012 ◽

Vol 107 ◽

pp. S105-S106

Author(s):

Taiwo Ngwa ◽

David Murray ◽

Ryan Law ◽

Suresh Chari

Keyword(s):

Elevated Serum ◽

Serum Igg4 Level ◽

Related Disease ◽

Igg4 Related Disease ◽

Poor Predictor ◽

Serum Igg4 ◽

Igg4 Level

Download Full-text

Prevalence of Elevated Serum IgG4 Level among Patients Diagnosed or Suspected with Cardiovascular Disorders

The Journal of Japanese College of Angiology ◽

10.7133/jca.17-00002 ◽

2017 ◽

Vol 57 (6) ◽

pp. 91-98 ◽

Cited By ~ 2

Author(s):

Nobukazu Ishizaka ◽

Yasunari Fujinaga ◽

Ryoto Hourai ◽

Tomohiro Fujisaka ◽

Koichi Sohmiya

Keyword(s):

Elevated Serum ◽

Cardiovascular Disorders ◽

Serum Igg4 Level ◽

Serum Igg4 ◽

Igg4 Level

Download Full-text

A Case of Immunoglobulin G4-Related Disease with Extensive Multiorgan Involvements

Case Reports in Rheumatology ◽

10.1155/2015/392893 ◽

2015 ◽

Vol 2015 ◽

pp. 1-9 ◽

Cited By ~ 3

Author(s):

Kazuhiko Higashioka ◽

Kenji Yoshida ◽

Kensuke Oryoji ◽

Kazuo Kamada ◽

Shinichi Mizuki ◽

...

Keyword(s):

Retroperitoneal Fibrosis ◽

Elevated Serum ◽

Clinical Findings ◽

Organ Involvement ◽

Immunoglobulin G4 ◽

Serum Igg4 Level ◽

Related Disease ◽

Igg4 Related Disease ◽

Serum Igg4 ◽

Igg4 Level

We report a case of IgG4-related disease (IgG4-RD) with multiple ten-organ involvement. This case showed many clinical findings, such as bilateral swelling of salivary and lacrimal glands, autoimmune pancreatitis, interstitial nephritis, retroperitoneal fibrosis, periaortitis, systemic swelling of lymph nodes, pulmonary lesions, splenomegaly, and jejunal lesions. He was suspected as having SLE or malignant lymphoma but diagnosed as having IgG4-RD by the elevated serum IgG4 level and histological findings from kidney and lymph node. We report a case of IgG4-RD with multiple ten-organ involvement that was successfully treated with prednisolone therapy.

Download Full-text